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Michael T. Lawton, Randall W. Porter, Joseph E. Heiserman, Ronald Jacobowitz, Volker K. H. Sonntag, and Curtis A. Dickman

✓ Thirty patients were treated surgically for spinal epidural hematoma (SEH). Twelve of these cases resulted from spinal surgery, seven from epidural catheters, four from vascular lesions, three from anticoagulation medications, two from trauma, and two from spontaneous causes. Pain was the predominant initial symptom, and all patients developed neurological deficits. Eight patients had complete motor and sensory loss (Frankel Grade A); six had complete motor loss but some sensation preserved (Frankel Grade B); and 16 had incomplete loss of motor function (10 patients Frankel Grade C and six patients Frankel Grade D). The average interval from onset of initial symptom to maximum neurological deficit was 13 hours, and the average interval from onset of symptom to surgery was 23 hours. Surgical evacuation of the hematoma was performed in all patients; 26 of these improved; four remained unchanged, and no patients worsened (mean follow up 11 months). Complete recovery (Frankel Grade E) was observed in 43% of the patients and functional recovery (Frankel Grades D or E) was observed in 87%. One postoperative death occurred from a pulmonary embolus (surgical mortality 3%). Preoperative neurological status correlated with outcome; 83% of Frankel Grade D patients recovered completely compared to 25% of Frankel Grade A patients. The rapidity of surgical intervention also correlated with outcome; greater neurological recovery occurred as the interval from symptom onset to surgery decreased. Patients taken to surgery within 12 hours had better neurological outcomes than patients with identical preoperative Frankel grades whose surgery was delayed beyond 12 hours. This large series of SEH demonstrates that rapid diagnosis and emergency surgical treatment maximize neurological recovery. However, patients with complete neurological lesions or long-standing compression can improve substantially with surgery.

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John A. Anson, Michael T. Lawton, and Robert F. Spetzler

✓ Dolichoectatic and fusiform aneurysms represent a small subset of cerebral aneurysms and are often among the most difficult to treat. A consecutive series of 40 patients with 41 of these two types of aneurysms is presented, including their clinical characteristics and surgical treatments. Common to all aneurysms was the pathological involvement of a length of blood vessel with separate inflow and outflow sites (nonsaccular). However, dolichoectatic aneurysms have markedly different symptoms and surgical treatments depending on their location in either the anterior or posterior circulation. Anterior circulation aneurysms involved the petrous internal carotid artery (ICA) in one, the supraclinoid ICA in three, the middle cerebral artery in 13, and the anterior cerebral artery in four patients. Posterior circulation aneurysms involved the basilar artery in 13, the vertebral artery in six, and the posterior inferior cerebellar artery in one patient. Various surgical procedures were performed, including direct clipping, trapping with bypass, proximal occlusion, resection with reanastomosis, transposition, aneurysmorrhaphy with thrombectomy, and wrapping. There was no surgical mortality in the patient series, and treatment was effective in many patients. Overall, outcome at late follow up was good (Glasgow Outcome Scale scores 1–2) in 78% of patients. Patients with anterior circulation aneurysms had better outcomes than patients with posterior circulation aneurysms, with good outcomes in 90% and 65% of the cases, respectively. Dolichoectatic and giant serpentine aneurysms may develop from smaller fusiform aneurysms and represent a spectrum of the same pathological entity. Arterial dissection may also play a role in the initial development of these aneurysms.

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Juvenile active ossifying fibroma

Report of four cases

Michael T. Lawton, Joseph E. Heiserman, Stephen W. Coons, Bruce D. Ragsdale, and Robert F. Spetzler

✓ Juvenile active ossifying fibroma is a rare lesion seldom seen by neurosurgeons. It originates in the paranasal sinuses during childhood, grows slowly, and encroaches on adjacent orbital and cranial compartments. In the past 3 years, four patients with this lesion were seen (three men and one woman; mean age 28 years). The clinical presentations were different with each patient: sinusitis, meningitis, periorbital pain, and a unique case of a juvenile active ossifying fibroma presenting with high-grade internal carotid artery stenosis and ischemic symptoms. Three patients were treated by transfacial approaches: two with a transfrontal—nasal approach and one with a transfrontal—nasoorbital approach. Two open resections resulted in gross-total excision and no recurrence as of the 2-year follow-up review. In the third patient, the tumor-encased carotid artery was preserved at the expense of a complete resection; that patient underwent superficial temporal artery—middle cerebral artery bypass and remains without ischemic symptoms or tumor recurrence at 2 years. The fourth patient underwent three subtotal endoscopic resections and is also without symptomatic recurrence at 2 years.

Three points must be made concerning these lesions. First, the clinical and radiographic characteristics of juvenile active ossifying fibroma may not be easily recognized by neurosurgeons, which could lead to misdiagnosis and mismanagement of these lesions. Second, this tumor can encase the carotid artery and cause severe stenosis or occlusion. Third, complete resection of the tumor is required to effect a cure, and transfacial approaches, which give wide exposure of the sinuses, appear to yield better, more radical resections than endoscopic procedures.

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Michael T. Lawton, Ronald Jacobowitz, and Robert F. Spetzler

✓ To investigate the role of angiogenesis in the pathogenesis of dural arteriovenous malformations (AVMs), 40 rats underwent common carotid artery—external jugular vein (CCA-EJV) anastomosis, bipolar coagulation of the vein draining the transverse sinus, and sagittal sinus thrombosis to induce venous hypertension. Fifteen rats underwent a similar surgical procedure, but venous hypertension was not induced. The 55 rats were divided into seven groups. Four groups, each containing 10 rats, underwent induced venous hypertension. The other three groups, each containing five rats, did not undergo induced venous hypertension. After 1, 2, or 3 weeks, dura mater was obtained from one group of hypertensive rats and from one group of nonhypertensive rats and was assayed for angiogenic activity (rabbit cornea bioassay). The remaining group of 10 hypertensive rats was not assayed to determine if sampling affected dural AVM formation. Unlike rats without CCA-EJV anastomosis, rats with CCA-EJV anastomosis had significantly increased postoperative sagittal sinus pressures (p < 0.0001). Mean angiogenesis indices were significantly greater in rats with venous hypertension than in rats without venous hypertension (p = 0.004). Dural AVMs formed in 42% of the 55 rats and facial AVMs formed in 51%. Angiogenic activity correlated positively with venous hypertension (ρ = 0.74). Development of dural AVMs correlated positively with both venous hypertension (p = 0.0009) and angiogenic activity (p = 0.04). These data indicate that venous hypertension may induce angiogenic activity either directly or indirectly by decreasing cerebral perfusion and increasing ischemia, and that dural AVM formation may be the result of aberrant angiogenesis.

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Randall W. Porter, Paul W. Detwiler, Robert F. Spetzler, Michael T. Lawton, Jonathan J. Baskin, Patrick T. Derksen, and Joseph M. Zabramski

Object. In this study the authors review surgical experience with cavernous malformations of the brainstem (CMBs) in an attempt to define more clearly the natural history, indications, and risks of surgical management of these lesions.

Methods. The authors retrospectively reviewed the cases of 100 patients (38 males and 62 females; mean age 37 years) harboring 103 lesions at treated a single institution between 1984 and 1997. Clinical histories, radiographs, pathology records, and operative reports were evaluated. The brainstem lesions were distributed as follows: pons in 39 patients, medulla in 16, midbrain in 16, pontomesencephalic junction in 15, pontomedullary junction in 10, midbrain—hypothalamus/thalamus region in two patients, and more than two brainstem levels in five. The retrospective annual hemorrhage rate was most conservatively estimated at 5% per lesion per year. Standard skull base approaches were used to resect lesions in 86 of the 100 patients. Intraoperatively, all 86 patients were found to have a venous anomaly in association with the CMB. Follow up was available in 98% (84 of 86) of the surgical patients. Of these, 73 (87%) were the same or better after surgical intervention, eight (10%) were worse, and three (4%) died. Two surgical patients were lost to follow-up review. Incidences of permanent or severe morbidity occurred in 10 (12%) of the surgically treated patients. The average postoperative Glasgow Outcome Scale score for surgically treated patients was 4.5, with a mean follow-up period of 35 months.

Conclusions. The natural history of CMBs is worse than that of cavernous malformations in other locations. These CMBs can be resected using skull base approaches, which should be considered in patients with symptomatic hemorrhage who harbor lesions that approach the pial surface. Venous anomalies are always associated with CMBs and must be preserved.

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Carlos A. David, A. Giancarlo Vishteh, Robert F. Spetzler, Michael Lemole, Michael T. Lawton, and Shahram Partovi

Object. This study was undertaken to evaluate the long-term angiographic outcome of surgically treated aneurysms, which is unknown. Specifically, the incidence of recurrent aneurysms, the fate of residual necks, and the de novo formation of aneurysms were evaluated.

Methods. One hundred two patients (80 females and 22 males; mean age 49 years; range 12–78 years) harboring a total of 167 aneurysms underwent late follow-up angiography; 160 aneurysms were surgically treated. Late angiographic follow-up review was obtained at a mean of 4.4 ± 1.6 years postsurgery (range 2.6–9.7 years). Late follow-up angiography revealed two recurrent aneurysms (1.5%) of 135 clipped aneurysms without residua. Of 12 aneurysms with known residua, there were eight “dog-ear” residua, of which two (25%) enlarged. One hemorrhage was noted, yielding a hemorrhage risk of 1.9% per year. A second subgroup with broad-based residua revealed dramatic regrowth in three of four cases. Eight de novo aneurysms were found in six patients, for an annual risk of 1.8% per year. A history of multiple aneurysms was associated with de novo aneurysm formation (p = 0.049, chi-square analysis).

Conclusions. This study confirms the long-term efficacy of aneurysm clip ligation. In addition, the authors found there is a small but significant risk of de novo aneurysm formation, particularly in patients with multiple aneurysms. Most residual aneurysm rests appear to remain stable, although a subset may enlarge or rupture. These findings support the rationale for late angiographic follow-up review in patients with aneurysms.

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G. Edward Vates, Kevin C. Wang, David Bonovich, Christopher F. Dowd, and Michael T. Lawton

✓ Bow hunter stroke, which is characterized by transient vertebrobasilar ischemia brought on by head turning, is an unusual condition usually caused by structural abnormalities at the craniocervical junction. The authors present a case in which compression of the left vertebral artery (VA) at the C4–5 level was caused by a laterally herniated intervertebral disc. A 56-year-old man presented with a 6-month history of dizziness and syncope when he turned his head 45° or more to the left. Transcranial Doppler (TCD) ultrasonography demonstrated decreased blood flow through the left VA, and angiography revealed an occlusion of the left VA at the C4–5 level, both when the patient turned his head to the left. Via an anterior cervical approach, the VA canal was unroofed through the transverse foramina to decompress the left VA at C4–5; intraoperatively, the left VA was found to be compressed by a laterally herniated cervical disc fragment. To the best of the authors' knowledge this is the first report of a laterally herniated cervical disc causing bow hunter stroke. The use of TCD may be of value in the diagnosis and management of the disorder, and herniated cervical disc must be included in the roster of potential causes for this rare disease.

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Matthew D. Smyth, Penny K. Sneed, Samuel F. Ciricillo, Michael S. Edwards, William M. Wara, David A. Larson, Michael T. Lawton, Philip H. Gutin, and Michael W. Mcdermott

Object. Stereotactic radiosurgery for arteriovenous malformations (AVMs) is an accepted treatment option, but few reports have been published on the results of this treatment in children. In this study the authors describe a series of pediatric patients with a minimum follow-up duration of 36 months.

Methods. From 1991 to 1997, 40 children (26 boys and 14 girls) with AVMs were treated with radiosurgery at the University of California at San Francisco (UCSF). Follow-up information was available for 31 children (20 boys and 11 girls) in whom the median age at initial treatment was 11.2 years (range 3.4–17.5 years). The median follow-up duration was 60 months (range 6–99 months). Sixteen percent of the AVMs were Spetzler—Martin Grade II; 68%, Grade III; 10%, Grade IV; and 6%, Grade V. The mean volume of the AVMs was 5.37 cm3 and the median volume was 1.6 cm3. The mean marginal dose of radiation was 16.7 Gy and the median dose was 18 Gy (range 12–19 Gy).

Angiography performed in 26 children confirmed obliteration of the AVM nidus in nine patients (35%), partial response in 16 patients (62%), and no response in one patient (4%). In five patients who refused angiography, magnetic resonance (MR) imaging revealed obliteration in two patients and partial response in three patients, bringing the overall obliteration rate associated with initial radiosurgery to 35%. Logistic regression analysis confirmed a significant correlation between marginal dose prescription and response (p = 0.025); in AVMs that received at least 18 Gy there was a 10-fold increase in the obliteration rate (63%) over AVMs that received a lower dose. Lesions smaller than 3 cm3 were associated with a sixfold increased obliteration rate (53%) over lesions larger than 3 cm3 (8%), but AVM volume was not a statistically significant predictor of response (p = 0.09). Twelve patients have since undergone repeated radiosurgery and are currently being followed up with serial MR imaging studies (in five cases, the AVM is now obliterated). During the follow-up period (1918 patient-months) there were eight hemorrhages in five patients, with a cumulative posttreatment hemorrhage rate of 3.2%/patient/year in the 1st year and a rate of 4.3%/patient/year over the first 3 years. There were two permanent neurological complications (6%) and no deaths in this study.

Conclusions. The lower overall obliteration rate reported in this series is most likely due to the larger mean AVM volumes treated at UCSF as well as conservative dose—volume prescriptions delivered to children. Significantly higher obliteration rates were observed when a marginal radiation dose of at least 18 Gy was delivered. The permanent complication rate is low and should encourage those treating children to use doses similar to those used in adults.